Anti-Site 2 protease/FITC Conjugated抗体上海钰博生物科技有限公司

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产品名称: Anti-Site 2 protease/FITC Conjugated抗体
英文名称: Anti-Site 2 protease/FITC
抗体货号: YB--19356R-FITC
产品规格: 20ul/50ul/100ul/200ul
级    别: , 分析纯, 见说明书
产品产地: 中国/美国
品牌商标: Ybscience
价    格: 2980元
抗原: KLH conjugated synthetic peptide derived from human Site 2 protease
抗原来源: Rabbit
抗体来源: Rabbit
适用物种: Human, Mouse, Rat, Cow, Horse, Sheep,
见说明书conjugate:见说明书
Isotype:见说明书
应用范围: 科研使用
更新时间: 2019/7/12 9:49:00
详细资料:  实验方法技术资料
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了解更多:进入公司展台
使用范围:仅限科研使用,不能应用于临床
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产品详细描述

 Anti-Site 2 protease/FITC Conjugated抗体

产品编号 YB-19356R-FITC
英文名称 Anti-Site 2 protease/FITC
中文名称 FITC标记的S2P内肽酶抗体
别    名 BRESEK; Endopeptidase S2P; IFAP; KFSDX; MBTP2_HUMAN; MBTPS2; Membrane bound transcription factor site 2 protease; membrane-bound transcription factor peptidase, site 2; membrane-bound transcription factor protease, site 2; Membrane-bound transcription factor site-2 protease; S2P; S2P endopeptidase; Site 2 protease; SREBPs intramembrane protease; Sterol regulatory element-binding proteins intramembrane protease.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 细胞生物  免疫学  泛素  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Cow, Horse, Sheep, 
产品应用 ICC=1:50-200 IF=1:50-200  
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 57kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Site 2 protease
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
产品介绍 background:
This gene encodes a intramembrane zinc metalloprotease, which is essential in development. This protease functions in the signal protein activation involved in sterol control of transcription and the ER stress response. Mutations in this gene have been associated with ichthyosis follicularis with atrichia and photophobia (IFAP syndrome); IFAP syndrome has been quantitatively linked to a reduction in cholesterol homeostasis and ER stress response.[provided by RefSeq, Aug 2009]

Function:
Intramembrane proteolysis of sterol-regulatory element-binding proteins (SREBPs) within the first transmembrane segment thereby releasing the N-terminal segment with a portion of the transmembrane segment attached. Site-2 cleavage comes after site-1 cleavage which takes place in the lumenal loop.

Subcellular Location:
Membrane. Cytoplasm.

Tissue Specificity:
Expressed in heart, brain, placenta, lung, liver, muscle, kidney and pancreas.

DISEASE:
Defects in MBTPS2 are the cause of ichthyosis follicularis-atrichia-photophobia syndrome (IFAPS) [MIM:308205]. A syndrome characterized by a peculiar triad of follicular ichthyosis, total or subtotal atrichia, and photophobia of varying degree. Histopathologically, the epidermal granular layer is generally well-preserved or thickened at the infundibulum. Hair follicles are poorly developed and tend to be surrounded by an inflammatory infiltrate. A subgroup of patients is described with lamellar rather than follicular ichthyosis. Non-consistent features may include growth and psychomotor retardation, aganglionic megacolon, seizures and nail dystrophy. Defects in MBTPS2 are a cause of keratosis follicularis spinulosa decalvans X-linked (KFSDX) [MIM:308800]. A rare disorder affecting the skin and the eye. Affected men show thickening of the skin of the neck, ears, and extremities, especially the palms and soles, loss of eyebrows, eyelashes and beard, thickening of the eyelids with blepharitis and ectropion, and corneal degeneration.

Similarity:
Belongs to the peptidase M50A family.

Database links:

Entrez Gene: 51360 Human

Entrez Gene: 270669 Mouse

Entrez Gene: 302705 Rat

Omim: 300294 Human

SwissProt: O43462 Human

SwissProt: Q8CHX6 Mouse

Unigene: 443490 Human

Unigene: 37577 Mouse

Unigene: 212224 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

生物在线声明:以上所展示的信息由企业自行提供,内容的真实性、准确性和合法性由发布企业负责。生物在线对此不承担任何保证责任。

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